Although being diagnosed with breast cancer is usually an extremely stressful experience for most women, a new study by researchers at Wake Forest Baptist Medical Center has found that there also can be unexpected benefits.

“Many women who have breast cancer often experience distress but sometimes are surprised that they also may experience a variety of positive outcomes following diagnosis,” said Suzanne Danhauer, Ph.D., associate professor of public health sciences at Wake Forest Baptist and lead author of the study.
The study, which is published in the current online edition of the journal Psycho-Oncology, examined change in post-traumatic growth (PTG) over two years in 653 women. PTG is defined as the positive psychological change experienced as a result of a struggle with highly challenging life circumstances. Commonly reported aspects of PTG include enhanced interpersonal relationships, increased appreciation for life, a sense of increased personal strength, greater spirituality and changes in life priorities or goals.
Participants completed surveys within eight months of diagnosis and also six, 12 and 18 months after that. The survey results were assessed using the Post-Traumatic Growth Inventory (PTGI), an instrument for assessing positive outcomes reported by persons who have experienced highly stressful or traumatic events.
According to the researchers, total PTGI scores increased over time mostly within the first few months following diagnosis. Greater PTGI scores were associated with education level, longer time since diagnosis, greater baseline level of illness intrusiveness and increases in social support, spirituality, use of active-adaptive coping strategies and mental health.
“Our findings suggest that there are women who see a variety of positive changes during and after breast cancer treatment,” Danhauer said. “Our study showed just how common it is for women to talk about the good things that have happened in their lives because of this illness, and it doesn’t seem to be related to how optimistic a person is or not.”
The study also showed that an increased amount of social support was associated with more post-traumatic growth in these women.
“Increased resources clearly help patients process what they are dealing with and feel supported, rather than feeling like they can’t talk about their illness,” Danhauer said. “For doctors and other health care providers, being open to hearing about what their patients are experiencing, including distress and unexpected positive outcomes, can be very beneficial.”

ALL is the most common form of cancer in children, with 3,000 children and young adults being diagnosed each year in the United States.

Dr. Offit hopes that ongoing research will also determine what percentage of childhood ALL patients have the PAX5 mutation. Current estimates suggest that it is rare. Additionally, the newly discovered gene mutation may someday help scientists determine how to target transcription factors to treat other non-inherited forms of leukemia where the PAX5 mutation is present.

“With a better understanding of the genetic elements that induce cancer susceptibility, or drive cancer to grow, we can more precisely target therapy as well as potentially prevent cancer from occurring in the first place,” added Dr. Offit.

Kenneth Offit, MD, MPH, Chief of the Clinical Genetics Service at Memorial Sloan-Kettering. “At the very least this discovery gives us a new window into inherited causes of childhood leukemia. More immediately, testing for this mutation may allow affected families to prevent leukemia in future generations.

» John M Corman

This article was first published in The Oncologist vol. 18 no.2,

As physicians, we are trained to expect loss. Every disease has the ability to develop, to progress, and ultimately to cause a patient to succumb. We protect ourselves from the pain of such an experience by steeling ourselves from the self-condemnation that accompanies wondering, “What more could I have done?” We are rarely able, however, to ever completely absolve ourselves.

We protect ourselves behind the armour of our white coats

As surgeons, we are fortified to deal with such events. We are invincible. We invade a body and put it back together. We are supermen. We make hundreds of critical, life-altering decisions before most people have had their morning coffee. Each of our decisions affects a patient’s life, both the quality and quantity. But how many are correct? Just one error, one poorly placed suture, one inaccurate assumption can result in disaster.

Just as disastrous is not feeling responsible or guilty after a complication or death. Our guilt, although perhaps self-indulgent, is redolent of our humanity. We employ every means to protect ourselves: defiance, arrogance, logic, and rationalisation – an alphabet of protective shields. Humour is a common mechanism for coping. However, the jokes are never particularly funny and are far from comforting.

We take classes to educate us in the stages of grief. We recognise the importance of the process for our patients and their families: the anger, the denial, and finally the acceptance. Although these stages are acceptable for others, we as physicians strive to remain stoic. By taking our feelings out of the equation, we somehow feel we are better able to help patients and their families through the ultimate emotional event. We effectively step back and isolate ourselves through the use of science and technology, protecting ourselves behind the armour of our white coats. To absorb every bit of grief that we observe during daily rounds would surely make our shoulders sag.

I was asked to see a patient by a colleague leaving for sabbatical. The patient was a vibrant, active, yet self-reflective man deteriorating daily from the effects of a massive pelvic tumour. Despite his wasting body, his eyes shone with his desire to do whatever possible to extend and improve his life. We entertained the idea of chemotherapy or perhaps palliative radiation treatments to buy him some time. In the end, he was simply too ill for either, but something had to be done. He was not ready to palliate.

We spent four weeks organising the surgical team for a heroic operation. It was an all-star team and six of us would operate: urologists, general surgeons, orthopaedists, and vascular surgeons. We spent hours reviewing anatomy, histology, and three-dimensional reconstructions. We developed a game plan, complete with contingencies against unanticipated finding. We sought perspectives from respected consultants and national thought leaders. We had every base covered.

Meanwhile, he spent the four weeks building his strength, maximising his nutritional status, and embracing his family. He spent time in meditation and prayer. His pain largely prevented mobility but could not dampen his spirit. Despite the weight loss, forfeiture of strength, and continually altered body image, he maintained a steely-eyed determination to beat the monster that grew within. He said that he would survive the cancer. He said he was an optimist and that, live or die, he was a winner.

We exhibited the typical surgical audacity that adrenaline fuels following his operation. With acknowledged conceit, we gave each other high-fives following his 10-hour surgery: “Couldn’t have gone better.” “That was a real surgery.” “One in a million.” We had completely removed the 5 lb sarcoma that was causing such pain. He had been bedridden and nauseated. Now, he’d be walking and eating within days. Perhaps our false bravado was emboldened by the knowledge that, although we may have been the victors in the battle, the war was not nearly over.

He spent the holidays at home, but the cancer came back. It was unremitting, vulgar, and obtrusive. We were going backwards. Despite the cancer’s recurrence, his family said that his illness had been an incredible spiritual journey. They were amazed by the outpouring from their community and had never loved or appreciated each other as they did during those intense three months.

He told me that he was not afraid of dying. He believed in an afterlife and that it was a beautiful place. He said that he was “just a bit concerned about the journey to get there.” If we are so hardened that such a loss is not profoundly felt, then we have indeed lost all compassion and with it every connection to humankind.

Despite our training, our medical armour, and our scientific approach to patient care, it is inevitable that some patients will permeate our shield and affect us in profound ways because we, too, are human. When they do, we must also grieve. Although our shoulders may sag if we allow ourselves to experience these emotions, we will rebound stronger and inspired to better care for our next patient.

June 7, 2013 — A new study of the use of genetic testing for cancer-causing mutations in affected families in France has found that its take-up is very low. Professor Pascal Pujol, Head of the Cancer Genetics Department, Montpellier University Hospital, Montpellier, France will tell the annual conference of the European Society of Human Genetics on June 9 that analysis of data from the French National Cancer Institute covering the years 2003 to 2011 showed that, although there had been a steady increase in tests performed for the breast and ovarian cancer-causing mutations BRCA1 and BRCA2, this was not the case with the MMR mutation, implicated in Lynch syndrome (a form of colorectal cancer). Only a third of relatives of individuals with either mutation underwent genetic testing themselves.

“Given that such testing can provide many options to enable individuals to manage their cancer risk, it is vital to encourage awareness and acceptance among both the public and medical professionals,” he will say. “For example, removal of the ovaries in women over 40 years old who carry a BRCA mutation decreases their overall cancer mortality by 20% and prophylactic mastectomy can reduce the chances of breast cancer in women carrying such a mutation by around 90%. Those who are unwilling to undergo prophylactic surgery can benefit from increased surveillance, with regular MRI (magnetic resonance imaging) scans. For familial colon cancer, screening by colonoscopy has been shown to decrease mortality. It is therefore regrettable that so few people seem to be aware of the benefits of genetic testing in families with a history of breast, ovarian, or colorectal cancer.”

Professor Pujol and colleagues from cancer centres across France analysed 240134 consultations and 134652 genetic tests from patients referred for a predisposition to breast or colorectal cancer. They found a substantial increase in tests for BRCA1/2 – from 2095 a year in 2003 to 7393 in 2011 — but for MMR mutations the increase was tiny — from 1144 to 1635 a year over the same period.

Mutations in BRCA1/2 genes are thought to be responsible for about 5% of all cases of breast and ovarian cancer. A woman with such a mutation has a risk of up to 87% of having breast cancer before she reaches the age of 80, as opposed to a risk of 8% in the general population. Such cancers are diagnosed at an average age of 43, as opposed to 60 in the general population, and are often more aggressive. In the case of ovarian cancer, a woman carrying a BRCA1 mutation has a risk of ovarian cancer of up to 63%.

Individuals with Lynch syndrome, or hereditary nonpolyposis colorectal cancer, have a 45% risk of developing colorectal cancer by the age of 70, and women with the syndrome are at increased risk of endometrial and ovarian cancers.

“While the increase in BRCA testing is encouraging, it is far from optimal. And the uptake of MMR testing for Lynch syndrome — responsible for 5% of all colorectal cancers — is frankly disappointing,” says Professor Pujol. “And of course, positive test results may have implications for other family members.

“While we have only studied the situation in France, we believe that our findings would be likely to be replicated in many other countries across the world. It is extremely worrying that such a simple test, which has the potential to spare whole families from devastating illness, is being so under-used. We urgently need a major programme of awareness among all those concerned, involving medical education and training, information programmes for patients and their families, public health campaigning, and improved genetic counselling,” he will conclude.

Every patient wants to be cured. But a culture that defines success as ‘cure’ condemns many patients and doctors to failure.

» Simon Crompton

What does treatment ‘success’ mean in cancer? Does it only mean curing the cancer? Or controlling it? Extending life? Or providing a good quality of life, even for a short time?

How we define success and failure is important because it has a profound impact on the goals that patients and their doctors set themselves and the experience of the cancer journey. Developing a shared understanding of what success means is also essential for informed public debate about the value of different interventions in different settings and how to get the best outcomes from the resources we have.

Roger Wilson, who has lived with sarcoma for 13 years, has pondered deeply on these issues. He says there is an urgent need for the cancer world to address the cultural influences that affect treatment decisions in advanced cancer: “We need to look at the patient demand for a ‘right to live’, the medical attitude that ‘success equals cure’ and the funder’s view that a dying patient is just a financial burden,” says Roger, who is a founder and President of Sarcoma UK.

Somewhere, amid these influences, what’s right for the individual can get lost.

Perspectives from patients and family on these issues provide a rich vein of insight for professionals and policy makers. In all their variety, they offer a central message: for a treatment to be ‘successful’ patients and their families must be properly engaged.Negotiating expectations
Kathy Oliver says that when her son Colin was diagnosed with a brain tumour in 2004 at the age of 24, her only measure of success was cure. “I didn’t know any better then,” says Kathy, who is co-director of the International Brain Tumour Alliance.

“When the diagnosis was given to us, we were sitting in a tiny room in a London hospital, but we may as well have been sitting on a planet in outer space. We had no map, no compass, no anchor to steady us. In our naïvety, we believed at that stage that treatment success could only be measured in terms of cure: we anticipated that neurosurgery would remove nearly all the visible tumour, followed by radiation that would eliminate every last cruel cancer cell, and then chemotherapy to guarantee a long and healthy life. Unfortunately, it didn’t work out like that.”

“As my son’s journey progressed, and his tumour’s level of malignancy did too, each successive treatment carried with it a different measure of success and expectation. With each treatment stage, the successes became more modest, but at the same time the availability of each treatment represented renewed hope.”

Annemie Spaak (not her real name) from Belgium, diagnosed with multiple myeloma in 2002 at the age of 37, tells a similar story of revising expectations. Since it is a disease “that doesn’t go away”, she says, patients often have a lasting relationship with their doctor, with treatment options being constantly discussed and renegotiated.

“My idea of success has definitely changed over time,” she says. “After my diagnosis, we discussed whether I should have more aggressive treatment which would extend life, or softer treatment that would give me better quality of life. At that stage, I was ready to go for the aggressive treatment – to go as close to a cure as was possible, because I had just given birth, had two young children, and I wanted to be with my family for as long as possible. But then I realised quite quickly that a cure was not going to happen, and once you accept this, you reset your definition of success. Now success means reaching certain milestones, to get the children through adolescence, and now to bring them to graduation.

“For someone over the age of 70, the objectives might be very different,” says Annemie, a board member of Myeloma Patients Europe. “And people late on the journey sometimes say they’re fed up with treatment and just want not to suffer and to be with their families.”

Expectations, and thus definitions of success, are also heavily shaped by cultural and social influences, says Luzia Travado, head of the psycho-oncology unit at the Champalimaud Cancer Centre in Lisbon, Portugal. Patients with advanced cancer who come from lower socio-economic groups tend to be more passive recipients of care, she says. Their expectations of treatment ‘success’ may be far less ambitious than better educated patients with higher incomes, who tend to want more control, and push more not only for a right to live but a right to a good quality of life.

What doctors do will be partly defined by this. Those working with higher socio-economic groups are more likely to propose active treatment towards the end of life.

“There are some patients who want to control, and some who are happy that the doctor controls,” she says. “But if you want properly responsive health systems, you have to keep asking people questions, whichever group they fall in, so that they can be involved if they want to. That doesn’t always happen.”

Buying time
The question of when active treatment should cease will always be difficult to negotiate, but with health services operating under ever tighter cost constraints, many patients now feel they are being denied a worthwhile shot at achieving a valuable added few weeks or months not because their expectations are unrealistic, but because they are considered unaffordable.

Many patients now feel they are being denied a shot at achieving a valuable added few weeks or months

Bettina Ryll, whose husband Peter died of melanoma in February last year after treatment in Sweden and the UK, is one among many representatives of cancer patients who worry that, despite high-level debate about ‘best’ treatment towards end of life, what actually happens is often dictated by economic considerations.

She has watched with interest as academics and policy makers have grappled with the cost of new cancer drugs, and she stands alongside the many patient groups who criticised the 2011 report of the Lancet Commission on cancer costs. This claimed that giving expensive care to patients during the last weeks of life is ‘futile’ and argued that too many of the new cancer treatments only extend life by a few weeks.

“Terminally ill people are members of society too,” says Bettina, who jointly founded the Melanoma Independent Community Advisory Board – an international network and resource for people affected by melanoma – in 2011. “They have paid into their health system, have made their contribution to their health care, and as a society we have a duty to honour that. I think it’s shocking to see how, suddenly, people who are no longer in the ‘healthy club‘ are considered not worthy of receiving any more from the health system.”

Bettina, who trained as a doctor herself, questions how far doctors really understand what a few extra weeks can mean to families, and she rejects the way active treatment tends to be counterposed to palliative care, arguing that treatments that extend life can also improve the quality of life.

“Peter’s melanoma was extremely aggressive,” she says. “It was diagnosed in February and by April the tumour had encased his whole arm so that he couldn’t move it and it was very painful. We didn’t expect him to see the summer.

“Then he went on a trial for a new drug and the tumour regressed – so much so, that he could even start rowing again. He died in February last year, so being on that drug bought us nearly a year. I remember thinking, before he went on the trial, ‘What’s the point of another month or so?’ But it gave us a chance to adjust, to say goodbye, to give our two daughters a chance to prepare, to get things in order. I think that year was the most valuable year of my life.”

“As healthy individuals, I think we underestimate the value of time for the person with cancer and their family. A month can be the equivalent of a year if you have limited life expectancy.”

Something to hope for
Kathy Oliver stresses that encouraging realistic expectations must be tempered with giving patients and their families something to hope for. If there is nothing to hope for there can be no hope of success.

“I wish that in the early days of my son’s diagnosis we had not faced such nihilism from some of the medical professionals we met,” she says.

“We often think of successful treatments that are either swallowed, injected, zapped or surgically performed. But to be given hope is just as important a treatment, and brings benefits not just for the patient, but for the family too. I cannot stress enough how important it is to maintain hope for patients facing devastating diagnoses.

“I know that in my son’s case, when there were no more surgeries, no more chemotherapies, no more radiation to be done, he still insisted that there was a plan for him. He kept receiving experimental therapy until the day he died, and in the last days kept reminding us not to forget to give him his treatment. Was the treatment futile in terms of medical benefit? Yes, it probably was. But what was important to my son, and also to us, was that there was a plan even towards the end.”

Of course, the experience of each family will be very different. Treatment plans will be influenced by the nature of the disease, its stage, and according to the character, socio-economic background, circumstances and wishes of the patient. With all those variables, doctors have a task on their hands when it comes to managing expectations while keeping hope alive.

Towards personalised measures of success
Roger Wilson says that a way forward is to provide doctors with “prognostic/risk assessment tools” that will give them the means to look at living with cancer in a rounded manner, not just in terms of medical treatments. This kind of personalised approach could yield a new integrated idea of treatment success for each patient.

“Such tools could be based on biological, behavioural, social and psychological markers: ‘this patient will do better if treated this way, another patient will need treating another way, and a third yet another way’ – even when clinically they are at the same stage with the same disease. Each treatment may involve lifestyle elements, would draw in expertise from non-cancer healthcare specialists, and would include practical support tuned to the needs of patients’ families.”

There have been tentative steps towards this kind of patient-centred research, he says, and it would sit very neatly with personalised cancer therapy approaches. “If we could reach the two objectives together, that would be a genuinely new definition of success.”

For Annemie Spaak a good relationship with their doctor remains the key for patients to perceive their treatment as successful. “It’s about partnership. I didn’t feel on the same wavelength with my first doctor and felt very unhappy, but when I changed I could accept my situation much better.”

However, a study published recently in the New England Journal of Medicine warns against jumping to the conclusion that a ‘good relationship’ necessarily improves the chances of patients achieving an outcome they perceive as ‘successful’. Quite the reverse in fact.

The surprise findings show that misunderstandings about treatments and their objectives are more common, not less, when doctors and patients have a good relationship.

The study, published in October last year, examined the expectations of 1193 patients receiving chemotherapy for metastatic lung or colorectal cancer. This can prolong life by weeks or months, or relieve symptoms, but does not cure. However, 69% of patients with lung cancer and 81% of those with colorectal cancer did not understand that chemotherapy was unlikely to cure their cancer. Surprisingly, perhaps, the risk of reporting inaccurate beliefs about the chemothe-rapy was higher among patients who rated their communication with their physician very favourably.

The implication is that the cost of a good relationship between doctors and their patients is an inability to face up to difficult facts – or at least a tacit agreement to collude in unrealistic expectations. The consequences of this may only come home to roost when patients and doctors are both faced with a sense of failure late in the cancer journey.

A planned and transparent transition
Bettina Ryll believes that altering the emphasis at medical school would go a long way. “At medical school you still have a rose-tinted view of how medicine saves lives, and maybe more could be done to demonstrate how palliative care is an important part of medicine too, and about the palliative ability of advanced treatments.”

Luzia Travado agrees that both patients and doctors find it difficult to acknowledge when cure is no longer possible. But it is up to the doctor to regulate expectations, right from the point of diagnosis. “It’s difficult,” she says. “Patients cling to any hope, and doctors want to avoid their patients getting too emotional. I’ve seen some patients who want to continue with their chemotherapy whatever the circumstances, because their coping mechanism is to not even consider the possibility of death.

“So it all depends on establishing a proper partnership and negotiating where you are heading at different stages. That’s why it’s so important that doctors are given the communication skills, and understand, for example, the SPIKES six-step protocol for delivering bad news.

“So it all depends on establishing a proper partnership, and negotiating where you are heading at different stages”

“Patients need to be helped to understand that the doctor can do something for them at all stages, even if they can’t cure. Here, we have abolished the phrase ‘There’s nothing more I can do for you.’ If there isn’t open communication from the start, patients and their families can easily feel frightened and isolated when the language doctors use changes, and doctors stop talking about ‘active’ treatment.”

Roger Wilson agrees with that prescription. But given the cultural influences that make it so difficult for doctors and their patients to look forward and discuss dying, he believes we have to look further than training time-strapped doctors. Healthcare systems need to plan for greater involvement from palliative care experts with psychological training from early on in the cancer journey.

“The truth is that we do not do communication well, but is unfair to look at it solely as a clinical problem best resolved by training cancer doctors better than we currently do,” he says. “Our healthcare systems have a general lack of will to support cancer patients with professionals who have had psychological training first and have then learned about cancer.

“A treatment approach which starts as curative but which recognises the ‘point of no return’ in a positive way would go a long way to challenging the current cultural influences on doctors and patients. The transition to palliative treatment should be planned and transparent. Expert palliative support should be seen as constructive and introduced to the patient long before there is the recognition that curative treatments are no longer feasible. Those who die will not die as ‘failures’, while those who are ‘cured’ will have had a better experience.”

Such planning could redefine everyone’s ideas of treatment success, he says. And it might mean that people with cancer and their families are helped to make decisions that are better suited to them as life reaches its end.

“It might mean that a few more patients die a few days earlier than they might otherwise have done, but the whole family experience and remembrance of dying would be more positive,” says Roger Wilson. “That would also be a benefit to society.”

Previous research has shown that women with larger social networks — including spouses or partners, female relatives, friends, religious and social ties, and ties to the community through volunteering — have better breast cancer survival. This study is among the first to show that the quality of those relationships also is important to survival.